Development of renal failure in children with the prune belly syndrome

Abstract

From 1970 to 1987, 32 male and female patients with the prune belly syndrome were evaluated. Of these patients 11 died as neonates or infants, and autopsy in 9 revealed 6 cases of diffuse and severe renal dysplasia. In the 10 surviving patients renal insufficiency developed and a total of 13 nephrectomies and renal biopsies were performed. Renal dysplasia was noted in 9 specimens, but unlike the infant kidneys, the dysplastic changes involved less than 25% of the parenchyma in most cases. Renal failure in these 10 patients was caused by pyelonephritis and obstruction. In our estimation perinatal renal failure in patients with the prune belly syndrome results from renal dysplasia caused by an in utero insult, while in older patients pyelonephritis and obstruction are the causes. Careful treatment of reflux, obstruction and urinary tract infections may decrease the incidence of renal failure in patients with the prune belly syndrome who survive infancy.